Guillain-Barré syndrome and hemophagocytic syndrome heralding the diagnosis of diffuse large B cell lymphoma: A case report

Table 1 Diagnostic criteria for diffuse large B cell lymphoma, Guillain-Barré syndrome and hemophagocytic syndrome

	Diffuse large B cell lymphoma	Guillain-Barré syndrome[3]	Hemophagocytic syndrome[4]
Diagnostic criteria	Diagnosis is based on WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues	Bilateral and flaccid weakness of limbs Decreased or absent deep tendon reflexes in weak limbs; Monophasic course and time between onset-nadir 12 h to 28 d; CSF cell count < 50/μL1; CSF protein concentration > normal value1; NCS findings consistent with one of the subtypes of GBS; Absence of alternative diagnosis for weakness	The diagnosis HLH can be established if either 1 or 2 below is fulfilled: (1) A molecular diagnosis consistent with HLH; and (2) Diagnostic criteria for HLH fulfilled five out of the eight criteria below. (A) Initial diagnostic criteria (to be evaluated in all patients with HLH); Fever; Splenomegaly; Cytopenias (affecting 2 of 3 lineages in the peripheral blood): Hemoglobin < 90 g/L (in infants < 4 wk: Hemoglobin < 100 g/L). Platelets < 100 × 109/L. Neutrophils < 1.0 × 109/L; Hypertriglyceridemia and/or hypofibrinogenemia: Fasting triglycerides 3.0 mmol/L (i.e., 265 mg/dL); Fibrinogen < 1.5 g/L; Hemophagocytosis in bone marrow or spleen or lymph nodes; No evidence of malignancy. (B) New diagnostic criteria; Low or absent NK-cell activity (according to local laboratory reference); Ferritin 500 mg/L; Soluble CD25 (i.e., soluble IL-2 receptor) 2400 U/mL

¹If colony-stimulating factor is not collected or results not available, nerve electrophysiology results must be consistent with the diagnosis Guillain-Barré syndrome.

NCS: Nerve conduction studies; GBS: Guillain-Barré syndrome; HLH: Hemophagocytic lymphohistiocytosis; CSF: Colony-stimulating factor.